History

Although desmoid tumors can arise in any skeletal muscle, they most commonly develop in the anterior abdominal wall and shoulder girdle.

Retroperitoneal neoplasms are more common in familial polyposis coli and Gardner syndrome after abdominal surgery than in other conditions.
[17]

Clusters of cases in families without evidence of any associated syndromes have also been reported.
[18]

A history of trauma (often surgical) to the site of the desmoid tumor is elicited in 1 in 4 cases.
[19] . Implant-associated breast desmoid tumors may occur.
[20]

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Physical Examination

Peripheral desmoid tumors

Peripheral desmoid tumors are firm, smooth, and mobile. They often adhere to surrounding structures. The overlying skin is usually unaffected. The presence of such a soft tissue growth should alert the clinician to delve more deeply into the family history for evidence of familial polyposis coli and Gardner syndrome. Extra-abdominal desmoid tumors are rare and may be first evident as gradually increasing leg swelling.
[21] Desmoid tumors may rarely appear on the foot.
[22]

Intra-abdominal and extra-abdominal desmoid tumors

Intra-abdominal desmoid tumors may be seen. Extra-abdominal desmoid tumors may also be seen (rarely) in the urological system, including in the bladder and scrotum.
[23, 24] Intra-abdominal desmoid tumors remain asymptomatic until their growth and infiltration cause visceral compression. Symptoms of intestinal, vascular, ureteric, or neural involvement may be the initial manifestations. An ethmoidal desmoid tumor has been described in a pediatric patient.
[25]

Breast desmoid tumors

Causes

The cause of desmoid tumors is uncertain and may be related to trauma or hormonal factors, or they may have a genetic association.

The familial polyposis gene on chromosome 5 has been extensively studied.
[5, 28]

An endocrine etiology is suggested. Desmoid tumors most commonly appear in young women during or after pregnancy. The tumors regress during menopause
[29] and after tamoxifen treatment.
[30] Desmoid tumors may regress after exposure to oral contraceptives.
[31]

The proliferative response of fibroblasts to estrogen has been established.
[32]

Tables

Contributor Information and Disclosures

Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Neil Shear, MD Professor and Chief of Dermatology, Professor of Medicine, Pediatrics and Pharmacology, University of Toronto Faculty of Medicine; Head of Dermatology, Sunnybrook Women's College Health Sciences Center and Women's College Hospital, Canada